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IDIOPATHIC HYPEREOSINOPHILIC SYNDROME OF THE HEART sgintmed.com | Hypereosinophilic syndrome. DermNet NZ dermnetnz.org | Attention Deficit Syndrome-ADHD Treatment-Attention Deficit Syndrome neuroassist.com | Syndrome X, Natural Approaches to the Metabolic Syndrome power-surge.com |
The hypereosinophilic syndrome (HS) is a disease characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause, with involvement of either the heart, nervous system, or bone marrow.[1] HS is a diagnosis of exclusion, after clonal eosinophilia (such as leukemia) and reactive eosionophilia (in response to infection, autoimmune disease, atopy, hypoadrenalism or cancer) have been ruled out. [2] There are some associations with chronic eosinophilic leukemia[3] as it shows similar characteristics and genetic defects.[4] If left untreated, HS is progressively fatal. It is treated with glucocorticoids such as prednisone.[2] The addition of the monoclonal antibody mepolizumab may reduce the dose of glucorticoids.[5]
[edit] ClassificationIn the heart, there are two forms of the hypereosinophilic syndrome, endomyocardial fibrosis and Loeffler's endocarditis.
[edit] Signs and symptomsAs HS affects many organs at the same time, symptoms may be numerous. Some possible symptoms a patient may present with include: [edit] DiagnosisNumerous techniques are used to diagnose hypereosinophilic syndrome, of which the most important is blood testing. In HS, the eosinophil count is greater than 1.5 × 109/L.[4] On some smears the eosinophils may appear normal in appearance, but morphologic abnormalities, such as a lowering of granule numbers and size, can be observed.[4] Roughly 50% of patients with HS also have anaemia.[4] Secondly, various imaging and diagnostic technological methods are utilised to detect defects to the heart and other organs, such as valvular dysfunction and arrhythmias by usage of echocardiography.[4] Chest radiographs may indicate pleural effusions and/or fibrosis[4], and neurological tests such as CT scans can show strokes and increased cerebrospinal fluid pressure.[4] A proportion of patients have a mutation involving the PDGFRA and FIP1L1 genes on the fourth chromosome, leading to a tyrosine kinase fusion protein. Testing for this mutation is now routine practice, as its presence indicates response to imatinib, a tyrosine kinase inhibitor.[8] [edit] TreatmentTreatment primarily consists of reducing eosinophil levels and preventing further damage to organs.[4] Corticosteroids, such as Prednisone, are good for reducing eosinophil levels and antineoplastics are useful for slowing eosinophil production.[4] Surgical therapy is rarely utilised, however splenectomy can reduce the pain due to spleen enlargement.[4] If damage to the heart (in particular the valves, then prosthetic valves can replace the current organic ones.[4] Follow-up care is vital for the survival of the patient, as such the patient should be checked for any signs of deterioration regularly.[4] After promising results in drug trials (95% efficiency in reducing blood eosinophil count to acceptable levels) it is hoped that in the future hypereosinophilic syndrome, and diseases related to eosinophils such as asthma and Churg-Strauss syndrome, may be treated with the monoclonal antibody Mepolizumab currently being developed to treat the disease.[5] If this becomes successful, it may be possible for corticosteroids to be eradicated and thus reduce the amount of side effects encountered.[5] [edit] EpidemiologyHS is very rare, with only 50 cases being noted and followed up in the United States between 1971 and 1982.[4] The disease is even more uncommon within the paediatric population.[4] Patients which lack chronic heart failure and those who respond well to Prednisone or a similar drug have a good prognosis.[4] However, the mortality rate rises in patients with anaemia, chromosomal abnormalities or a very high white blood cell count.[4] [edit] References
[edit] External links
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